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Doing well

Student doesn’t let illness stop her from achievement

Posted: Sunday, March 26, 2006

 

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  Summer Engler of Sterling recently received the Parasca Science Research Award at the University of Alaska Anchorage for her academic achievement. The junior doesn't let her struggle with lupus keep her from her studies. Photo by Phil Hermanek

Summer Engler of Sterling recently received the Parasca Science Research Award at the University of Alaska Anchorage for her academic achievement. The junior doesn't let her struggle with lupus keep her from her studies.

Photo by Phil Hermanek

A common symptom among people diagnosed with lupus is extreme fatigue.

In Sterling resident Summer Engler’s case, the diagnosis instead has strengthened her resolve to study harder.

The auto immune disorder causes the body’s immune system to over-produce antibodies and actually fight healthy cells and tissues.

Engler was first diagnosed with the disease two years ago and in spite of her disability, the University of Alaska Anchorage junior has been named the first recipient of the Parasca Science Research Award for undergraduates.

“The treatments (for the disease) make it harder to study, but I study harder now that I have the choice,” Engler said at her family’s home off Feuding Lane, where she lives with her parents, Gary and Lori Engler, and sisters, Megan, 20, and Samantha, 16.

Engler was first diagnosed with lupus in February 2004.

She was admitted to the Intensive Care Unit of Providence Alaska Medical Center in Anchorage with peridarditis, an inflammation around the heart.

That’s when doctors discovered systemic lupus erythematosus.

 

Engler and friend, Ryan Jager, share a moment at the Engler home.

Photo by Phil Hermanek

To bring down the inflammation, Engler received high doses of steroids and to treat the lupus, she received low-dose chemotherapy. She was kept in the hospital for a week.

One week after being released, however, she was admitted again.

Engler’s diaphragm muscles were so weak, she was having trouble breathing.

“They found I had myasthenia gravis — an auto immune neuromuscular disease,” she said. “My immune system is attacking my muscles and making me weak.”

She spent the next four months at Providence.

Her friend of three years, Ryan Jager, stayed at her bedside for most of one month, and now serves as her personal care assistant.

“I was just on the cusp of needing to be intubated because the muscles were almost too weak (for me) to breathe,” Engler said.

Now, in addition to receiving steroid treatments for the lupus, she undergoes monthly five-hour infusions of donor antibodies — intravenous immuno globulin — through a port in her chest.

Although the treatments make it more difficult for her to study, she says she is determined and the prognosis is that she can live a normal life.

Since she was 16, and a student at Skyview High School, Engler has wanted to be a doctor.

Because her mom and several aunts also have lupus, she has long had an interest in internal medicine — specifically rheumatology at this point in her life.

As a biological sciences student in the honors program at UAA, Engler was taking a class with Dr. Jocelyn Krebs, and asked if she could do research with the doctor.

 

Engler visits with her father, Gary Engler, in the family home off Feuding Lane in Sterling.

Photo by Phil Hermanek

The honors program requires participants to take classes in such topics as ethics and culture, in addition to regular course work, and requires students to complete an honors thesis in order to graduate as an honors grad.

“Dr. Krebs said she had some topic questions I could work on,” Engler said.

One of the topics was the maintenance of copper homeostasis, or the balance of copper in human cells, which became the subject of the research proposal for which Engler received the Parasca Award.

The award carries with it up to $4,000 for research project expenses and a $1,000 student stipend for the recipient.

In her research project abstract, Engler explains the objective of her research:

“Copper is essential for life in all cells, but high levels of this co-factor can become toxic. Therefore, cells must maintain a balance between copper uptake and copper export.

“Genetic defects in copper metabolism can be lethal and may cause diseases such as Menkes disease (copper deficiency) and Wilson’s disease (copper overload).”

Menkes disease, Engler states, is caused by the loss of protein involved in copper export from cells.

In this disease, copper in the diet is absorbed normally into intestinal cells, but these cells then fail to export this copper for delivery throughout the body, so that most tissues are starved for copper. The disease is generally fatal by three to four years if untreated.

Wilson’s disease is also a defect of copper transport, according to Engler. Normally, excess copper in the body is sent to the liver, which then transports it into bile for excretion.

In Wilson’s disease, the liver fails to secrete copper into the bile, leading to toxic levels of copper in liver cells, causing liver damage and the release of excess copper back into the bloodstream, damaging the eyes, brain and kidneys. If untreated, the disease results in liver failure, severe brain damage and death, Engler states.

Engler will research copper homeostasis in the yeast Saccharomyces cerevisiae under the supervision of Dr. Krebs. The proteins involved in regulating copper levels within the cell are similar in yeast and human, and yeast cells are easy to grow in the laboratory.

Engler’s research may offer a better understanding of copper imbalance diseases in humans such as Menkes and Wilson’s.

“I’m trying to figure out what went wrong to make this imbalance cycle happen,” she said.

While her research will not result in a cure for either Menkes or Wilson’s disease, Engler said she hopes the project “may open the way to greater insights involving copper homeostasis.”

Her research will extend through the spring 2007 semester and the results will be presented at the Spring 2007 Undergraduate Research and Discovery Symposium.

Because of her medical challenges, Engler has constant muscle weakness and sometimes uses her walker on campus.

As a result, she has gained an understanding for the obstacles faced by students with disabilities and she has become a member of CAPRA, the College Access Project for Rural Alaska.

Her advocacy work for the disabled, ability to overcome her own disabilities and personal achievement have also earned her the Muscular Dystrophy Association’s 2006 Personal Achievement Award for Alaska.

She will appear on the Jerry Lewis MDA Labor Day weekend telethon in September.

When asked how she manages to achieve so much despite her medical challenges, Engler said, “I try to always keep a positive attitude.

“In the hospital, I tried to be goofy with the doctors and nurses,” she said.

“While at Providence, my doctors couldn’t tell me if I’d ever walk again. I can.”



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